• 제목/요약/키워드: Congenital defects

검색결과 300건 처리시간 0.027초

Congenital Cleft of Anterior Arch and Partial Aplasia of the Posterior Arch of the C1

  • Choi, Ji-Won;Jeong, Je-Hoon;Moon, Seung-Myung;Hwang, Hyung-Sik
    • Journal of Korean Neurosurgical Society
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    • 제49권3호
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    • pp.178-181
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    • 2011
  • Congenital anomalies in arches of the atlas are rare, and are usually discovered incidentally. However, a very rare subgroup of patients with unique radiographic features is predisposed to transient quadriparesis after minor cervical or head trauma, A 46-year-old male presented with a 2-month history of tremor and hyperesthesia of the lower extremities after experiencing a minor head trauma. He said that he had been quadriplegic for about 2 weeks after that trauma. Radiographs of his cervical spine revealed bilateral bony defects of the lateral aspects of the posterior arch of C1 and a midline cleft within the anterior arch of the atlas. A magnetic resonance imaging revealed an increased cord signal at the C2 level on the T2-weighted sagittal image. A posterior, suboccipital midline approach for excision of the remnant posterior tubercle was performed. The patient showed significant improvement of his motor and sensory functions. Since major neurologic deficits can be produced by a minor trauma, it is crucial to recognize this anomaly.

Epidemiological survey on the relationship between Agent Orange / Dioxin exposure and reproductive anomalies and congenital deformities of inhabitants living in A Luoi valley, Thua thien hue province

  • Hung, Tran-Manh
    • 한국환경보건학회:학술대회논문집
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    • 한국환경보건학회 2003년도 Challenges and Achievements in Environmental Health
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    • pp.26-26
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    • 2003
  • During the Viet Nam war, A Luoi located in Thua Thien Hue Province was heavily sprayed herbicides by the Ranch Hand Operation of U.S.A force (549.274 gallons). After 30 years of knocking the operation off, Dioxin has still contaminated on environment and human body in A Luoi valley. The results of the study show that there is an epidemiological relationship between AO/Dioxin exposure and increasing rate of reproductive anomalies and birth defects (the rate of reproductive anomalies including congenital deformities after the war during 10-year and 25-year is higher than that before the war from 1.5 to 4 times). The study found that the highest rate of reproductive anomalies is spontaneous abortion. The average is premature death and congenital deformities, and the lowest rate is dead foetus and hydratidiform mole. The results of the primary assessment suggested that Dioxin transport from environment to human body through the food chain exposed by Dioxin such as fish, chicken, duck. A mitigation plan for residual dioxin contamination from Agent Orange Hervicides is requeste indispensable to inhabitants in Aluoi District.

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Needs for Post-hospital Education among Parents of Infants and Toddlers with Congenital Heart Disease

  • Lee, Bo Ryeong;Koo, Hyun Young
    • Child Health Nursing Research
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    • 제26권1호
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    • pp.107-120
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    • 2020
  • Purpose: This study was conducted to investigate the educational needs of parents of infants and toddlers with congenital heart disease (CHD) after hospital discharge. Methods: Qualitative content analysis was conducted of in-depth interviews of eight parents, and the results of an online survey of 171 parents were analyzed quantitatively. Results: Only 16.4% of parents reported that they had received education after hospital discharge on how to provide care for a child with CHD at home. The main reason why parents did not receive education on this topic was that they did not have sufficient opportunities or information (75.5%). In addition, 97.1% of parents stated that they needed educational programs that would be available at home after discharge. In terms of specific educational content, parents expressed the highest needs for education on the symptoms of CHD and ways to cope with them, the prognosis of CHD, and the growth and development of infants and toddlers with CHD. Conclusion: The study showed that parents' educational needs were high in many ways. However, the information and educational opportunities offered after discharge were insufficient compared to those needs. Further research is needed to develop post-hospital educational programs that meet their needs.

선천성 갑상선기능저하증과 가성부갑상선기능저하증을 동반한 환자의 전신마취 하 치과치료 (General Anesthesia for Dental Treatment in the Congenital Hypothyroidism and Psuedohypoparathyroidism)

  • 서광석;신터전;김현정;장주혜
    • 대한치과마취과학회지
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    • 제13권3호
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    • pp.139-143
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    • 2013
  • The patient who has congenital hypothyroidism and pseudohypoparathyroidism could have mental retardation even though adequate hormone treatment and cannot endure conventional dental treatment. In this case, general anesthesia is selected to administer effective dental treatment. But, there could be symptoms such as anemia, neuropathy, associated pituitary or adrenal hypofunction, cardiac failure even in euthyroid state. And, bradycardia, mental dullness, hypothermia, slow reflexes can appear in case of inadequate thyroid hormone replacement. Especially, macroglosssia, slow drug metabolism, exaggerated responses to anesthetic agents and decreased ventilatory responses could be problem during general anesthesia. The presentation of hypoparathyroidism also varies depending on the chronicity of the result of hypocalcemia. Muscle spasms/tetany, paresthesias, and seizures may occur in an acute onset. Chronic hypocalcaemia causes fatigue, muscle cramps, lethargy, personality changes, and cerebration defects.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization

  • Kylat, Ranjit I;Witte, Marlys H;Barber, Brent J;Dori, Yoav;Ghishan, Fayez K
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제22권6호
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    • pp.594-600
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    • 2019
  • With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Genetics of kidney development: pathogenesis of renal anomalies

  • Hahn, Hye-Won
    • Clinical and Experimental Pediatrics
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    • 제53권7호
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    • pp.729-734
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    • 2010
  • Congenital anomalies of the kidney and urinary tract (CAKUT) account for more than 50% of abdominal masses found in neonates and involve about 0.5% of all pregnancies. CAKUT has a major role in renal failure, and increasing evidence suggests that certain abnormalities predispose to the development of hypertension and cardiovascular disease in adulthood. To understand the pathogenesis of human renal anomalies, understanding the development of kidney is important. Diverse anomalies of the kidney corresponding to defects at a particular stage of development have been documented recently; however, more research is required to understand the molecular networks underlying kidney development, and such an investigation will provide a clue to the therapeutic intervention for CAKUT.

Crystalloid Cardioplegic Solution과 Blood Cardioplegic Solution을 사용한 선천성 심기형 환자에서의 술 후 심기능 평가에 대한 비교 연구 (A Comparative Study of the Postoperative Cardiac Performance after Repair of Congenital Heart Defects with Crystalloid and Blood Cardioplegic Solution)

  • 김용진;김영태
    • Journal of Chest Surgery
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    • 제27권10호
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    • pp.815-823
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    • 1994
  • This study was undertaken to hemodynamically determine the differences of myocardial protective effect between crystalloid and blood cardioplegic solution. Twenty nine children undergoing cardiac operations due to cyanotic congenital heart diseases were randomized into two groups receiving crystalloid or blood cardioplegia. Cardiac indices and other hemodynamic datum were examined postoperatively. Although there was no statistical differences between groups, postoperative stroke volume indices and left ventricular stroke work indices were slightly better with blood cardioplegia. We also found that postoperative left atrial pressures[p=0.0003], central venous pressures[p=0.004], and heart rates[p=0.014] were significantly lower with blood cardioplegia. The fact that relatively lower ventricular preloads [left atrial pressure and central venous pressure] were required to provide adequate cardiac output in blood cardioplegia group suggested superior myocardial protective effect of blood cardioplegic solution.

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Persistent Atrial Fibrillation Related to a Congenital Pericardial Defect and Left Atrial Appendage Herniation

  • Lee, Heemoon;Jeong, Dong Seop;Kim, In Sook;Park, Byung Jo
    • Journal of Chest Surgery
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    • 제48권6호
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    • pp.429-431
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    • 2015
  • Congenital pericardial defects (CPDs) are infrequent anomalies that are usually asymptomatic and are discovered incidentally during unrelated interventions. Here we report the case of a CPD with herniation of an enlarged left atrial appendage identified during total thoracoscopic ablation (TTA) for persistent atrial fibrillation (AF). The persistent AF was successfully treated with a hybrid procedure, in which TTA was followed by an electrophysiological study.

성인의 선천성 심혈관 기형에 대한 수술 성적 (The Surgical Outcome of Congenital Cardivascular Disease in Adult)

  • 김영대
    • Journal of Chest Surgery
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    • 제28권4호
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    • pp.340-345
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    • 1995
  • Between 1983 and 1993, 250 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular disease were reviewed. 222 patients were divided into acyanotic group and 28 patients were cyanotic group. The most common defects were atrial septal defect [96 patients and ventricular septal defect [95 patients . There were 128 patients in the third decade, 71 patients under 20 years of age, 40 patients in the fourth decade and 11 patients over 40 years of age. The male to female ratio was 1.05:1. Operative mortality was 6.8% [4.1% in the acyanotic group and 26.8% in the cyanotic group and the most common cause of death was low cardiac output syndrome.

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정중흉골절개하 경심낭적 기관지 흉막누공의 폐쇄 치험 1예 (Transpericardial closure of postpneumonectomy bronchopleural fistula -Report of a case-)

  • 김주현;이재원
    • Journal of Chest Surgery
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    • 제19권3호
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    • pp.528-533
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    • 1986
  • Total anomalous pulmonary venous connection is relatively rare cyanotic congenital heart diaease, which represents 1-4% of all congenital cardiac defects. Generally in the majority cases, severe heart failure and cyanosis develops in the early infancy. Because of high mortality in the untreated infants and surgical risk, there are still many things to be improved. Two patients with total anomalous pulmonary venous connection are presented, which we recently experienced. The one was 10 year old female with supracardiac type drained through left innominate vein, and survived the operation and continuous to do well for 1 year. The other 5 year old female with mixed type (right pulmonary vein drained via coronary sinus and left pulmonary vein through left innominate vein) was operated successfully under hypothermia and extracorporeal circulation, and followed up for 6 months without problem. It was very rare case in the literature.

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